Surgical Management of Congenital

(Non-Ebstein) Tricuspid Valve Regurgitation

Sameh M. Said,a Harold M. Burkhart,b and Joseph A. Dearanic

Congenital tricuspid valve regurgitation (TR) is a relatively uncommon condition that

includes a heterogeneous group of lesions with a unique management strategy. There are
wide anatomic variations that lead to congenital TR in patients without Ebstein malforma-
tion. Possible etiologies may include primary valve abnormalities (eg, congenital absence
of chordae) or other forms of tricuspid valve dysplasia as in congenitally unguarded
tricuspid valve, and patients with pulmonary atresia and intact ventricular septum, which
can be similar to Ebstein’s valves or secondary regurgitation in association with other
anomalies as in atrioventricular septal defects, right ventricular outflow tract obstructive
lesions (pulmonary stenosis or atresia with ventricular septal defect [VSD]), tricuspid valve
annular dilatation in association with right ventricular volume overload lesions as in
congenital coronary arterial fistula with secondary right ventricular enlargement, and Uhl’s
anomaly. Iatrogenic etiologies in the congenital population include TR secondary to pre-
vious VSD closure (chordal or leaflet injury), pacemaker or internal cardiac defibrillator
lead-induced TR, and traumatic TR (ruptured chordae). Presentation depends on the
severity of the disease and may be apparent in infancy, childhood, or adulthood.
Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann 15:46-60 © 2012 Elsevier Inc. All
rights reserved.

Anatomic and Tricuspid Valve Dysplasia

In general, there are two categories of TV dysplasia (Table 1);
Surgical Considerations those with and those without downward displacement. Cases

T he tricuspid valve has a complex anatomic structure

composed of three well-developed leaflets: chordae ten-
dinae, papillary muscles, and the tricuspid annulus. The
right atrium and right ventricle (RV) play an important role in
that complex structure as well (Fig. 1).1-9 TV competency
depends on successful interaction between these compo-
nents.
The 3-dimensional complex structure of the TV annulus
differs from that of the “saddle-shaped” mitral annulus. It is
also a dynamic structure that changes its shape in relation to
the cardiac cycle (Fig. 2A-B).10 All these facts need to be
considered during TV repair.11
aDivision of Cardiovascular Surgery, Mayo Clinic, Rochester, MN.
bAssociate Professor of Surgery, Division of Cardiovascular Surgery, Mayo
Clinic, Rochester, MN.
cProfessor of Surgery, Division of Cardiovascular Surgery, Mayo Clinic,
Rochester, MN.
Address correspondence to Joseph A. Dearani, MD, Division of Cardiovas-
cular Surgery, Mayo Clinic, 200 First St. SW, Rochester, MN 55905;
E-mail: jdearani@mayo.edu
with downward displacement, which is caused by failure of
delamination of valve leaflets from the underlying myocar-
dium, are by definition, Ebstein malformation (EM). Al-
though downward displacement is often given as the sole
feature of EM, dysplasia of the leaflets and subvalvar appara-
tus are an almost universal feature as well. The pathognomic
findings of EM are downward displacement caused by failure
of delamination, whether or not features of dysplasia are
present, and a myopathy of the RV that is also a result of
failure of delamination. When downward displacement is
absent, then the anatomic entity is referred to as “tricuspid
valvular dysplasia.”12
Echocardiography confirms the diagnosis, determines the
degree of tricuspid valve regurgitation (TR), allows accurate
evaluation of the tricuspid leaflets and subvalvar apparatus
(displacement, tethering, dysplasia, etc.), size and function of
the right and left ventricles, and detection of septal defects.
Magnetic resonance imaging (MRI) is being increasingly used
in all types of patients with cardiac disease, including those
with EM and other forms of congenital TR. Functional assess-
ment can be made including quantitative measurements of
left and right ventricular size and function (Fig. 3). At pres-

46 1092-9126/12/$-see front matter © 2012 Elsevier Inc. All rights reserved.

doi:10.1053/j.pcsu.2012.01.009
Non-Ebstein tricuspid regurgitation 47

Table 1 Etiologies of Congenital Tricuspid Regurgitation

Primary valvar abnormalities
With Displacement ⴝ Ebstein’s anomaly
Without Displacement ⴝ Non-Ebstein’s (Tricuspid Valve
Dysplasia)
TV dysplasia in pulmonary atresia with intact septum
(PA/IVS)
Congenitally unguarded TV
Uhl’s anomaly
Secondary valvar abnormalities
Atrioventricular septal defect (AVSD)
Right ventricular outflow tract obstruction (RVOTO)
Tetralogy of Fallot (TOF)
Pulmonary atresia with ventricular septal defect (PA/
VSD)
Pulmonary stenosis
Tricuspid regurgitation (TR) with VSD
Gerbode defect
TV annular dilatation (Volume overload lesion)
Coronary artery fistula
Atrial septal defect
Figure 1 Pathologic specimens showing tricuspid valve with dys- Partial anomalous pulmonary venous connection
plastic leaflets but no displacement. TV, tricuspid valve. (Reprinted (PAPVC)
with permission from the Mayo Clinic Foundation for Medical Ed- Iatrogenic TR
ucation and Research.). Detachment of TV leaflet during VSD closure
Pacemaker/Cardioverter-defibrillator

ent, we use echocardiography (2- and 3-dimensional) for

evaluation of TV anatomy and MRI for assessment of right
and left ventricular size and function.
TR in the Setting of Pulmonary
Atresia with Intact Interventricular Septum
Patients with pulmonary atresia and intact ventricular septum
(PA/IVS) tend to have a higher incidence of a regurgitant TV.
The mechanism of this regurgitation is different from patients
with tetralogy of Fallot (TOF). In TOF, TR is usually caused by
right ventricular dilatation with secondary annular dilatation
from long-standing pulmonary regurgitation. The degree of TR
reflects the degree of right ventricular dilation in these cases, and
the progression of TR has been an indication to proceed with
pulmonary valve replacement (PVR). In PA/IVS, the TV is usu-
ally dysplastic (Fig. 4) and may require surgical intervention
from the beginning. In addition, reoperation for persistent or
recurrent TR is almost inevitable in this group of patients, and
the likelihood of a successful repair and re-repair will decrease
with time. The application of various valvuloplasty techniques
that have been learned with other TV abnormalities (eg, cone
technique for EM) has been helpful in optimizing successful
tricuspid valvuloplasty. The addition of a bidirectional cavopul-
monary anastomosis (BDCPA) (ie, 1.5 ventricle repair) to the
tricuspid valvuloplasty can be considered when there is hyp-
oplasia of the RV or significant RV dysfunction to avoid or delay
valve replacement. A single-ventricle pathway can be consid-

Figure 2 (A) The reconstructed ring shape for tricuspid annuloplasty based on the average results obtained in healthy
subjects at the time of minimum tricuspid annulus area. The positive x-y-z axis indicates the respective directions
toward the septum, the posterior wall, and the right atrium. The average of each of the manually selected TA locations
is indicated by the white dot. A, anterior; L, lateral; P, posterior; S, septum; TA, tricuspid annulus. (B) Dynamic changes
in the tricuspid valve annulus during cardiac cycle. (Reprinted with permission.)
48 S.M. Said, H.M. Burkhart, and J.A. Dearani

Figure 3 Left: Three-dimensional reproduction of heart with Ebstein malformation demonstrating: views of tricuspid
valve from (A) short axis and (B) axial imaging. Right: Cardiac MRI showing systolic and diastolic contours of functional
RV and atrialized portion of RV in (A,B) axial and (C,D) short-axis views. (E) Severity index representing ratio of areas
of right atrium and atrialized RV in numerator and summation of functional RV and left atrium and left ventricular areas
in denominator (ie, severity index ⫽ [right atrial area ⫹ atrialized right ventricular area]/[functional right ventricular
area ⫹ left atrial area ⫹ left ventricular area]). (F) Degree of apical displacement of septal leaflet of tricuspid valve (in
millimeters) measured in ventricular diastole. aRV, atrialized right ventricle. fRV, functional right ventricle; LA, left
atrium; LV, left ventricle; RA, right atrium; TVAL, tricuspid valve anterior leaflet; TVPL, tricuspid valve posterior leaflet;
TVSL, tricuspid valve septal leaflet. (Reprinted with permission.)

ered as an alternative to two right-sided valve replacements,
particularly when the RV is on the smaller side.
Tricuspid Regurgitation in Association
with Right Ventricular Outflow Tract
Obstruction (TOF, Pulmonary Stenosis
or PA with Ventricular Septal Defect)
The free pulmonary regurgitation that results from relief of right
ventricular outflow tract obstruction, as an essential component
of the complete surgical repair of TOF, pulmonary stenosis, and
PA with ventricular septal defect (VSD), can result in volume
overload with secondary right ventricular dilatation and the de-
velopment of functional TV regurgitation.13 TR can also result
from leaflet distortion associated with VSD closure in such cases.
PVR is indicated in this group of patients in the presence of
symptoms, RV volume overload, or the development to arrhyth-
mias (atrial or ventricular). Concomitant TV repair at the time of
PVR is still controversial because it is unclear the effect of PVR
alone on RV size and secondary TR. In the series reported by
Kogon et al,14 the authors analyzed 35 patients who underwent
PVR subsequent to repair of TOF or congenital pulmonary ste-
Non-Ebstein tricuspid regurgitation 49

arrhythmias and the progression of ventricular dysfunction

and right-sided heart failure. The importance of continued
follow-up for all patients on a regular basis cannot be over-
emphasized so that the timing of reoperation is optimized. In
general, we prefer a flexible ringed annuloplasty for tricuspid
repair when somatic growth is complete and utilize eccentric
annuloplasty techniques in earlier childhood.
Tricuspid Regurgitation in Association with VSD
VSD is one of the most frequently encountered congenital
cardiac anomalies. TR in association with VSD can result
from various mechanisms which may include: (1) leaflet de-
formity, (2) TV overriding or straddling, (3) abnormal
chordal attachments (Fig. 5), (4) clefts in or between the
tricuspid valve leaflets, (5) Gerbode defect, (6) leaflet distor-
tion secondary to patch closure of VSD, and (7) TV detach-
Figure 4 Intraoperative photo showing dysplastic tricuspid valve in
ment. Detachment of the TV septal and/or anterior leaflet is
the setting of PA with IVS. (Reprinted with permission from the
Mayo Foundation for Medical Education and Research.) sometimes required to facilitate VSD closure, which in turn
may reduce the chance of residual shunt or heart block. The
concern with this technique is related to the potential for TV
dysfunction and regurgitation, but the data are few and long-
nosis in the period between 2002 and 2008. These patients had
term outcomes related to the TV function are unknown. The-
at least moderate TR at the same time of their PVR. The degree of
oretical impairment of the TV annulus in the long term may
TR as well as RV dilatation decreased significantly at 1 month
also exist, which can result in valvular stenosis.18 Bol-Raap et
postoperatively, with no further improvement at late follow-up.
al19 reported a series of 188 patients who underwent VSD
We analyzed the outcomes of 53 patients who had surgical
closure between 1992 and 2001. Temporary detachment of
treatment of pulmonary valve stenosis between 1951 and
the TV was performed in 46 patients (24%). Trivial TR was
1982.15 In 28 patients, there were 35 re-interventions, which
present postoperatively in 10 patients (22%) among the de-
included PVR for free pulmonary regurgitation in 21 patients,
tachment group, and in 57 patients from the non-detach-
open valvotomy in five, and pulmonary balloon valvuloplasty in
ment group (40%). No patient developed tricuspid stenosis.
three for residual pulmonary valve stenosis, closure of atrial
Mean follow-up was 2.6 years, and no patient required re-
septal defect in two, right ventricular outflow tract reconstruc-
operation for TV dysfunction. Although controversial, the
tion in one, closure of iatrogenic VSD in one, ligation of aorto-
study reported by Tatebe et al20 recommended avoidance of
pulmonary fistula in one, and tricuspid valve annuloplasty with
detachment of the TV in small infants and those with Down
simultaneous coronary artery bypass grafting in one patient. In
syndrome because of the high incidence of TR. However, we
addition, atrial and ventricular arrhythmias were common, oc-
agree with Bol-Raap et al that VSD closure can be performed
curring in 20 patients (38%). Severe pulmonary valve regurgi-
tation, although well tolerated initially, has deleterious effects on
RV systolic function and exercise capacity over time. In addition,
evidence suggests that long-standing pulmonary regurgitation
with right-sided cardiac chamber enlargement may be associ-
ated with the development of arrhythmias and sudden death.16
In our practice, we have used the development or progression of
TR related to annular dilation as an indication for PVR. In addi-
tion, it is our policy to repair the TV when TR is graded moderate
or more. Importantly, the decision to repair the TV should be
made preoperatively because the conditions of anesthesia gen-
erally reduce the grade of TR by at least one grade.
There were a total of 446 reoperations for conduit failure in
370 patients in the period from 1964 to 2001.17 Progression
of TR is an indication for conduit replacement in this group of
patients. We have a low threshold for tricuspid valve repair
under these circumstances, and it is considered at the time of
the first conduit change or concomitant with PVR. TV repair
represented 5.7% of the total associated procedures that were
performed at the initial time of the conduit operation during Figure 5 Pathologic specimen showing abnormal chordal attachment
the same time period.17 Progression of TR is considered as a (arrow) of the tricuspid valve in relation to the VSD. TV, tricuspid valve;
marker of the progression of conduit dysfunction and the VSD, ventricular septal defect. (Reprinted with permission from the
need for surgical intervention to prevent the occurrence of Mayo Foundation for Medical Education and Research.)
50 S.M. Said, H.M. Burkhart, and J.A. Dearani

Figure 6 (A) Cardiac CT scan with 3D reconstruction showing giant right coronary artery aneurysm (black arrow) in the
setting of congenital right coronary artery-to-coronary sinus fistula. (B) Cardiac MRI showing partial anomalous
pulmonary venous connection of the right upper pulmonary vein to the superior vena cava. In A or B, the tricuspid
valve annulus is usually dilated secondary to right ventricular enlargement and volume overload. RCA, right coronary
artery; PAPV, partial anomalous pulmonary vein; SVCL, superior vena cava. (Reprinted with permission of the Mayo
Foundation for Medical Education and Research.)

safely with a very low complication rate, and TV detachment
does not result in an increased incidence of TR.
The Gerbode Defect
Left ventricular-to-right atrial shunts represent less than 1%
of congenital heart diseases.21 In 1958, Franke Gerbode re-
ported the first series of successful repairs of such anoma-
lies.22 The septal leaflet of the TV divides the membranous
septum into interventricular and atrioventricular (AV) por-
tions. The defect can occur in any or both of these portions;
the septal leaflet of the TV is commonly involved. If the shunt
is in the interventricular segment of the membranous sep-
tum, it can result in significant TR. This often requires repair
at the time of VSD closure.
Tricuspid Regurgitation Secondary to Annular
Dilatation and Right Ventricular Volume Overload
Congenital coronary arterial fistulae (Fig. 6A), atrial septal defect,
and partial anomalous pulmonary venous connection (Fig. 6B) rep-
resent examples of congenital lesions that are characterized by
large left-to-right shunt. The resultant large RV volume overload
leads to RV enlargement with secondary TV annular dilatation.
The end result may be the development of significant TR.
TR Secondary to Implantable Permanent
Pacemaker or Cardioverter-Defibrillator Leads
The use of permanent pacemakers (PPM) and implantable
cardioverter-defibrillators (ICD) in the congenital population

Figure 7 Pathologic specimens (A-B) and intraoperative photo (C) showing perforation or entanglement of the tricuspid valve
leaflet with pacemaker or cardioverter-defibrillators lead (arrows). (Reprinted with permission from the Mayo Clinic.)
Non-Ebstein tricuspid regurgitation
is increasing because both atrial and/or ventricular arrhyth-
mias are a frequent late complication.
Consequently, TR secondary to these devices are increas-
ing. The mechanisms of TR (Fig. 7A-C) are related to: (1)
leaflet perforation by the lead; (2) lead entanglement of the
TV apparatus; (3) lead impingement of the TV leaflets; and
(4) lead adherence to the TV. In our experience, we analyzed
the records of 571 consecutive patients with non-Ebstein TR
who underwent TV operation for severe TR in the period
between 1993 and 2010. Twenty-five patients (4%) had se-
vere TR secondary to PPM or ICD lead placement. TV re-
placement was performed in 14 patients (56%), while it was
repaired in the remaining 11 patients (44%).
Indications for Surgery
TR has been considered a benign lesion for a long time, but
recent studies suggest that irrespective of pulmonary artery
pressure or left ventricular ejection fraction, TR negatively
affects long-term survival.23 The reported 2-year postopera-
tive event-free survival in the presence of severe preoperative
RV dysfunction is 57%.24 Severe TR induces chronic RV vol-
ume overload, which leads to progressive ventricular dilata-
tion, dysfunction, and eventually right-sided heart failure.
Timely correction of TR will preserve RV function, improve
functional capacity, and improve long-term survival. TV re-
pair is the preferred treatment strategy when it is feasible,
particularly in children. Optimal timing is now recom-
mended before the onset of RV dysfunction, even in asymp-
tomatic patients.25,26
The indications for surgery (summarized in Table 2) in TR
include symptoms or cyanosis (when atrial septal defect is pres-
ent), decreased exercise tolerance, progressive cardiomegaly on
chest x-ray, progressive RV dilatation or reduction of RV systolic
function by echocardiography, or appearance of atrial or ven-
tricular arrhythmias. In borderline situations, the echocardio-
graphic determination of high probability of TV repair makes
the decision to proceed earlier with operation easier.
Progression of TR is taken into consideration for timing of
intervention for PVR as well (Table 2). In general, we repair
the TV when the degree of TR is moderate or more at the time
of PVR. It is important to make the decision for TV interven-
tion on preoperative outpatient echocardiography rather
than on intraoperative transesophageal echocardiography
because of the difference in hemodynamics under general
anesthesia.
Surgical Techniques
Tricuspid Valve Repair
The goal of the operation is repair of the TV. To increase
the number of successful TV repairs, particularly in chil-
dren, we have been using a cone-type reconstruction (Fig.
8A-J).27,28 This results in 360° of tricuspid leaflet tissue
surrounding the right AV junction. This allows leaflet tis-
sue to coapt with leaflet tissue, similar to what occurs with
normal TV anatomy. The hinge point of the reconstructed
TV is at the true TV annulus (AV junction). Modifications
51
Table 2 Indications for Surgery
A. Tricuspid Regurgitation
Symptoms or reduced exercise intolerance
Cyanosis (concomitant interatrial communication)
Progressive RV dilatation
Progression of RV dysfunction
Onset, progression of arrhythmias
B. Pulmonary Valve Replacement
Symptoms of right heart failure and exercise intolerance
RV systolic pressure > 2/3 of systemic blood pressure
RVEDVI > 150 ml/m2
Onset, progression of arrhythmias
Prolongation of QRS complex > 180 msec or > 3.5
msec/year
Progressive RV dysfunction
Progression of TR
include suturing the base of the intact papillary muscle on
the RV free wall to the ventricular septum (Sebening
stitch) at the appropriate level with interrupted, pledgeted
mattress sutures (Fig. 9).29 It is important to place these
mattress sutures deep into the ventricular septum and that
the anterior leaflet has been thoroughly mobilized (delam-
inated). Alternatively, a monocusp-type repair based on a
large anterior leaflet has also been successful.30,31 It is im-
portant that the repair is tension free. To facilitate this,
augmentation of the mid-anterior leaflet with an ellipse of
autologous pericardium or other pliable synthetic material
as CorMatrix (Fig. 10) can increase the height of the ante-
rior leaflet, which facilitates coaptation with the ventricu-
lar septum while minimizing tension at an inferior annu-
loplasty line. Importantly, we avoid the creation of
complete pericardial leaflets (from annulus to leading
edge) because of poor durability. When inferior leaflet
tissue is absent, this area is often the site of residual regur-
gitation, but this area can be bridged with a piece of au-
tologous pericardium (Fig. 11A-B). If no septal leaflet is
found, we perform the surgical delamination of the ante-
rior and inferior leaflets as usual, and then the papillary
muscle(s) is mobilized and sutured to septum. The ante-
rior leaflet is rotated clockwise toward the septum, and the
inferior leaflet is sutured to the septum beyond the coro-
nary sinus instead of the septal leaflet. In case of complete
tethering of the leading edge of the leaflet (ie, linear at-
tachment; Fig. 12A-B), surgical fenestrations are per-
formed distally, which effectively result in the develop-
ment of chordae (Fig. 12C). Annular plication sutures,
purse-string annuloplasty, or eccentric commissuroplasty
can be performed to further narrow the tricuspid annulus.
A flexible annuloplasty C-shaped ring is our preference and
is used routinely when somatic growth allows. This begins
at the anteroseptal commissure and ends at the inferosep-
tal commissure (adjacent to the coronary sinus).
The addition of an edge-to-edge stitch, thus creating a
double orifice TV, can be added to the above maneuvers to
optimize coaptation and improve valve competency in selected
circumstances provided iatrogenic stenosis is avoided. Closure
52 S.M. Said, H.M. Burkhart, and J.A. Dearani

Figure 8 Operative steps for the “cone technique.” (A) The first incision is made with a no. 15 blade in the anterior
leaflet at 12:00; the incision is a few millimeters away from the true annulus. The incision is then extended
rightward in a clockwise fashion using a scissors. It is common for there to be a true space between the anterior
leaflet and the RV in this region (ie, normally delaminated leaflet). However, when the transition is met between
the anterior and inferior (posterior) leaflets, it is common for there to be failure of delamination (inset), resulting
in fibrous and muscular attachments between the leaflet and myocardium. The diagram demonstrates the scissors
approaching the area where there is some adherence of leaflet tissue to the underlying myocardium. The
dissection continues in a way that a portion of distal anterior leaflet and some inferior leaflet tissue is “surgically
delaminated.” The most important aspect of this surgical delamination is to incise all fibrous and muscular
attachments between the body of the leaflet and the right ventricular myocardium, but to maintain intact all
fibrous and (and occasionally muscular) attachments of the leading edge of the leaflet to the underlying myocar-
dium. Importantly, do not disrupt chordal attachments to the leading edge of any leaflet. (B) As the anterior and
surgically delaminated inferior leaflet is reflected away from the right ventricular myocardium, all fibrous and
muscular attachments into the body of the underside of the leaflet are incised as shown with the scissors. It is
important to keep all attachments of the leading edge of the leaflet intact; if the edge is linearly attached, then
surgical fenestrations are created distally. The dotted triangle represents the atrialized RV. (C) Dissection is
continued with a scissors, with the goal of taking down all attachments between the septal leaflet and myocardium
but preserving all attachments of the leading edge to the endocardium, as described above. The dissection should
proceed medially all the way to the anteroseptal commissure. The leaflet tissue is typically very fragile and thin in
this area. There can be marked variability in the status of the leading edge of the septal leaflet as was described for
the anterior and inferior leaflets. If there is a linear attachment, then surgically created fenestrations are also made
in this leaflet (not shown). (D) Intraoperative photo demonstrating the mobilized anterior and inferior (posterior)
leaflets. Natural fenestrations are shown at the junction of the anterior and inferior leaflets (arrows). LV, left
ventricle; RA, right atrium; RV, right ventricle. (Figure continues.)
Non-Ebstein tricuspid regurgitation 53

Figure 8 (E) After the anterior, inferior, and septal leaflets have been completely mobilized, the cut edge of the inferior leaflet
is rotated clockwise to meet the proximal edge that has been prepared of the septal leaflet. The two are approximated with
interrupted 6-0 monofilament sutures completing the cone reconstruction. This results in 360° of leaflet tissue that will make
up the new tricuspid valve orifice. (F) After the cone reconstruction is completed, the atrialized RV (RV) is examined to
determine if plication is necessary. Note the position of the right coronary artery (RCA) in the true tricuspid valve annulus and
keep in mind that there are acute marginal branches of the right coronary artery that can also be compromised with plication.
This figure demonstrates the technique for internal plication of the atrialized RV. Monofilament 4-0 or 5-0 is utilized and the
suture is begun distally, ie, closest to the apex of the RV. It is important to frequently examine the outside of the inferior wall
of the RV to insure that inadvertent compromise of branches of the right coronary artery is avoided. (G) The suture line is
advanced toward the base of the heart, ie, toward the AV groove. As the dotted lines of the triangle are effectively approxi-
mated, the atrialized RV is excluded. Occasionally, the suture line extends beyond the AV groove onto the right atrium. Care
is taken to avoid compromise of the right coronary artery. After the sides of the triangle are approximated, the entrance into
the excluded atrialized segment of the RV is then closed to eliminate the “blind pouch.” (H) After the plication or resection
is completed, the newly constructed tricuspid valve is then reattached below the level of the true tricuspid annulus (ventric-
ular side of conduction tissue). Because the neotricuspid valve will have an orifice that is smaller than the original dilated AV
junction, a plication of the inferior annulus is necessary to meet the size of the neotricuspid valve. The inferior annulus is
usually plicated with two to four simple or figure-of-eight 5-0 monofilament sutures. Proper placement of these sutures is
critical to the success of this repair. The sutures need to be deep enough to maintain the integrity of the new annulus intact without
compromising or significantly distorting the right coronary artery. If the size discrepancy between the true tricuspid annulus and the
neotricuspid valve is large, smaller annular plication sutures should be placed in multiple areas around the true annulus to avoid
distortion of the right coronary artery by a large plication in a single area. To avoid heart block, the suture line is deviated caudad to
the membranous septum and AV node, which is marked by the previously mentioned vein and fatty tissue. (Figure continues.)

of intra-atrial shunts is performed routinely when operation is
performed beyond infancy. Right reduction atrioplasty is rou-
tinely performed, and the maze procedure is added when atrial
tachyarrhythmias are present.
In cases of TR secondary to VSD closure, there is often
disrupted commissural chordae (Fig. 13A) at the anteroseptal
commissure with or without annular dilatation. Treatment
consists of approximating the adjacent edges of the leaflets at
this commissure together, thus obliterating that commissure.
In the presence of annular dilatation, a flexible annuloplasty
ring can be added to remodel the annulus as described above
(Fig. 13B).
In the presence of AV septal defect (Fig. 14A-B), closure of
the residual cleft in the left AV valve is performed, and place-
ment of annuloplasty rings on each valve is routine. In se-
lected situations, leaflet augmentation with pericardium or
54 S.M. Said, H.M. Burkhart, and J.A. Dearani

Figure 8 (I) The completed cone reconstruction of the tricuspid valve. Saline is injected via bulb syringe into the RV to
examine competency of the tricuspid valve. Any residual fenestrations or areas of leak are repaired as needed. A subtotal
closure of the patent foramen ovale or atrial septal defect is usually performed. If it is believed that a bidirectional Glenn
shunt is needed because of a small effective orifice of the neotricuspid valve, or because of severely depressed RV
function, then the intra-atrial communication may be closed completely. Redundant right atrium is excised from each
side of the atriotomy and then the atriotomy is closed. RCA, right coronary artery. (Inset; ring annuloplasty to reinforce
the repair). (J) The completed cone reconstruction of the tricuspid valve for Ebstein’s anomaly. This represents an
“anatomic repair” because there is 360° of tricuspid leaflet tissue that surrounds the orifice of the tricuspid valve that is
anchored at the level of the normal right AV junction (true tricuspid valve annulus). Extreme forms of thinned,
atrialized RV are plicated and redundant right atrium is excised. (Reprinted with permission of Mayo Foundation for
Medical Education and Research.)

other prosthetic material is used to increase the height of the
attenuated leaflet.
In the presence of TR induced by PPM or automatic
implantable cardioverter defibrillator (AICD) leads, repair
techniques vary and depend on the degree of damage of
leaflet(s). In the absence of extensive TV leaflet damage,
valve repair is preferred and usually involves: (1) remov-
ing (incising) the lead away from the damaged leaflet; (2)
suture repair of the leaflet defect if present; or (3) reposi-
tioning the lead by suture fixation in the recess of either
the infero-septal or antero-inferior commissure (Fig. 15A-
C). Ringed annuloplasty is performed. We confirm the
resolution or improvement of the TR with intraoperative
transesophageal echocardiography.
Tricuspid Valve Replacement
Bioprosthetic TV replacement remains a good alternative for
the treatment of TR when valve repair is not feasible, partic-
ularly in adults. Porcine bioprosthetic valve replacement is
preferred because of relative good durability of the porcine
bioprosthesis in the tricuspid position and the lack of need
for chronic warfarin anticoagulation. Pericardial bioprosthe-
ses should be avoided in the tricuspid position because of
relatively stiffer leaflets and poor durability in this position.
Mechanical TV replacement should be considered rarely and
only in selected circumstances because there is a higher fre-
quency of prosthetic valve dysfunction (thrombosis) com-
pared with mechanical valves in other cardiac positions, par-
ticularly when right ventricular function is poor and disc
mobility may be reduced.
When TV replacement is required, care is taken to preserve
the conduction tissue and avoid injury of the right coronary
artery. The conduction tissue is usually identifiable by a small
vein traversing an area of yellow (fat) tissue adjacent to the
membranous septum. Before replacing the TV, we resect the
portion of the anterior leaflet toward the RV outflow tract to
avoid potential RV outflow tract obstruction after TV replace-
Non-Ebstein tricuspid regurgitation 55

Figure 9 Diagram showing the Sebening stitch in which the base of the intact papillary muscle on the right ventricular
free wall is moved toward the ventricular septum at the appropriate level with interrupted, pledgeted mattress sutures.
It is important to place these mattress sutures deep into the ventricular septum. In addition, it is important to insure
thorough mobilization of the anterior leaflet to avoid dimpling of the right ventricular free wall (inset). (Reprinted with
permission from the Mayo Clinic.)

ment. In EM, the suture line is deviated toward the atrial side In cases of TV leaflet damage secondary to PPM/AICD
of the true annulus and membranous septum to avoid injury leads, tricuspid valve replacement is performed as described
to the AV node. Posteriorly, the suture line is typically in the above for non-Ebstein congenital TR. The decision to remove
atrial septum (often incorporating the edge of the atrial septal the endocardial lead and place an epicardial system is indi-
defect patch) (Fig. 16A-B). To avoid injury to the right coro- vidualized. In circumstances when there are excellent thresh-
nary artery (Fig. 17), the anterior suture line is deviated ceph-
alad to the true annulus, where the smooth and trabeculated
portions of the atrium meet each other. The bioprosthesis is
oriented so the recess between the struts straddles, the mem-
branous septum, and conduction tissue. We prefer tying the
valve sutures while the heart is beating to detect any rhythm
abnormalities. The position of the coronary sinus relative to
the prosthesis depends on the distance between it and the AV
node. The coronary sinus is left draining into the right atrium
when there is sufficient distance between it and the conduc-
tion tissue. When the coronary sinus is in close proximity to
the conduction tissue, the prosthesis is positioned cephalad
to it so that the coronary sinus drains below the prosthesis
into the RV.
In non-Ebstein congenital TR, all sutures are placed in
native anterior and inferior TV leaflet tissue circumferen-
tially. The posterior suture line is typically placed through
septal leaflet tissue at the level of the AV groove when suffi- Figure 10 Intraoperative photo showing the augmented anterior tricus-
cient septal leaflet is present; if there is a lack of septal leaflet, pid valve leaflet with cor matrix membrane, which increases the height
suture placement is as described above for EM. Care is taken of the anterior leaflet, and facilitates coaptation with the ventricular
for accurate suture placement at the anteroseptal commis- septum and or septal leaflet while minimizing tension at an inferior
sure, a common site for periprosthetic leak or injury to the annuloplasty line. Importantly, we avoid the creation of complete peri-
conduction system resulting in heart block. cardial leaflets (from annulus to leading edge) because of poor durability.
56 S.M. Said, H.M. Burkhart, and J.A. Dearani

Figure 11 (A-B) When inferior leaflet tissue is absent or septal leaflet is diminutive, this area can be bridged with a piece
of autologous pericardium to avoid iatrogenic tricuspid stenosis. Interrupted sutures are often used to avoid a “purse
string effect.” (© 2011 Mayo Clinic.)

olds in the endocardial system and there is significant tricus-
pid annular dilatation, the ventricular lead can be maintained
intact and positioned lateral to the sewing ring of the biopros-
thesis. It is important to be sure the lead is freely mobile
alongside the prosthesis.
In general, postoperative management includes short-term
(3 months) warfarin anticoagulation for porcine bioprosthe-
ses and life-long aspirin, 81 mg daily. When a mechanical
valve is used, the target INR is 3 to 3.5 in addition to aspirin,
81 mg daily.

Figure 12 In case of complete tethering of the leading edge of the leaflet, ie, linear attachment (A,B), surgical fenestra-
tions are performed distally that effectively result in the development of chordae (C). (Reprinted with permission from
the Mayo Clinic.)
Non-Ebstein tricuspid regurgitation 57

Figure 13 In cases of tricuspid regurgitation secondary to VSD closure, there is often disrupted commissural chordae (A) at the
anteroseptal commissure with or without annular dilatation. Treatment consists of approximating the adjacent edges of the leaflets
at this commissure together, thus obliterating that commissure. In the presence of annular dilatation, a flexible annuloplasty ring
should be added to remodel the annulus as described above (B). (Reprinted with permission from the Mayo Clinic.)

Additional oxide, intra-aortic balloon counter pulsation (especially in

Perioperative Strategies
The following operative and postoperative strategies should
be considered following correction of TR, particularly when
there is significant RV dysfunction: (1) measures that de-
crease RV afterload and pulmonary artery pressure, eg, nitric
adult patients), manipulation of mechanical ventilation with
the PCO2 goal of 30 to 35 mmHg; (2) cautious volume ad-
ministration with a CVP goal of ⬍12 mmHg; (3) temporary
atrial pacing to achieve faster heart rate of 100 to 120; (4)
minimize transfusions; and, finally, (5) delayed chest closure.
Inotropic support while separating from bypass usually in-

Figure 14 In the presence of atrioVSD (A), closure of the residual cleft in the left AV valve is performed, and placement of
annuloplasty rings on each valve is routine (B). In selected situations, leaflet augmentation with pericardium or other prosthetic
material is used to increase the height of the attenuated leaflet (not shown). (Reprinted with permission from the Mayo Clinic.)
58 S.M. Said, H.M. Burkhart, and J.A. Dearani

Figure 15 (A-C) In the presence of pacemaker or cardioverter defibrillator leads, repair techniques vary and depend on
the degree of damage of leaflet(s). In the absence of extensive leaflet damage, valve repair is preferred and usually
involves: removing (incising) the lead away from the damaged leaflet; suture repair of the leaflet defect if present; or
repositioning the lead by suture fixation in the recess of either the infero-septal or antero-inferior commissure, and
ringed annuloplasty is performed. (Reprinted with permission from the Mayo Clinic.)

Figure 16 Diagram (A) with the corresponding intraoperative photo (B) showing the valve suture line (dotted line) is
placed on the atrial side of the membranous septum and AV node to avoid injury to the conduction system. The suture
line is also deviated cephalad to the tricuspid annulus posterolaterally when the tissues are thin, to avoid injury to the
right coronary artery. Anteriorly, the suture line is deviated cephalad to the junction of the smooth and trabeculated
portions of the right atrium. This results in the prosthesis being mounted in an intra-arterial position. When there is
sufficient distance between the coronary sinus and the AV node (marked by the vein of D), the coronary sinus is left
draining normally into the right atrium. If the coronary sinus and the conduction tissue are in close proximity, the
suture line is deviated further into the right atrium, leaving the coronary sinus to drain below the prosthesis into the RV
(not shown). The sutures are placed and tied with the heart perfused and beating to ensure that normal AV conduction
is preserved. CS, coronary sinus. (Reprinted with permission from the Mayo Clinic.)
Non-Ebstein tricuspid regurgitation
cludes a combination of epinephrine and milrinone infu-
sions. Low doses of vasopressin infusion are often helpful
because systemic vasoplegia and low peripheral vascular re-
sistance are commonly present with right-sided heart failure
related to TR and/or pulmonary regurgitation. The mecha-
nism of this is not completely understood but may relate to
preoperative afterload-reducing agents such as ace inhibitors,
or may be related to hepatic congestion, or hormonal abnor-
malities related to atrial dilatation.
The rate of weaning inotropic support in the postoperative
period is related to the degree of RV dysfunction. Inotropic
support can be weaned relatively quickly when RV dysfunc-
tion is mild to moderate and early extubation is the rule.
When there are significant degrees of RV dysfunction, the
inotropic wean should be slow because of the expected drop
in the cardiac output that can occur between 6 to 12 hours
postoperatively.
Although there are no data that afterload reducing agents
are helpful for RV dysfunction (in contrast to left ventricular
dysfunction), it is common to use them and usually includes
ace inhibitors. In addition, beta blockers are also commonly
added because of evidence of improvement with left-sided
dysfunction. The duration of medical therapy is determined
by the primary Cardiologist and is guided by serial echocar-
diography, exercise testing, etc. In selected circumstance,
sildenafil is used for 1 to 3 months when there is known
elevation in pulmonary artery pressures with or without se-
vere RV dysfunction. Antiarrhythmic therapy is used as indi-
cated by rhythm abnormalities.
The Mayo Clinic Experience
From 1993 to 2010, we performed 1,204 procedures for
congenital TR. Congenital non-Ebstein tricuspid valve was
the etiology in 571 patients. TV repair was the main proce-
Figure 17 Pathologic specimen showing the close relation between
the right coronary artery and the tricuspid valve annulus. The right
coronary artery (long arrow) can be easily kinked (short arrow) dur-
ing tricuspid valve repair procedure. RCA, right coronary artery;
TV, tricuspid valve. (Reprinted with permission from the Mayo
Clinic.)
59
Table 3 Etiology of TR in 571 Patients with Non-Ebstein Tri-
cuspid Valve
Diagnosis Percentage
TOF/PA with VSD 31
VSD 15
AVSD 14
Transposition of great arteries 8
Truncus arteriosus
Double outlet right ventricle
Permanent pacemaker 4
Cardioverter-defibrillator
Congenital, other 28
dure in 458 patients, at a mean age of 29, while in tricuspid
valve replacement was performed in 113 patients (mean age
of 40). Table 3 summarizes the different diagnoses for the
571 patients. Repeat sternotomy (ⱕ 3) was required in 81%
of the TV repair group versus 65% in the TV replacement
group, while four sternotomies or more were performed in
8% of the TV repair group versus 34% of the TV replacement
group. Early mortality in the repair group was 2.4%, while in
the replacement group it was 9.7%.
Summary
The wide and infinite variability of anatomic abnormality
with EM and congenital TV dysplasia demonstrate that every
valve is a little different and no two hearts are alike. . .illus-
trating why this lesion continues to be one of the most chal-
lenging valve lesions for the surgeon. There have been more
reports in the literature of tricuspid valvuloplasty techniques
for EM and congenital tricuspid dysplasia than any other
valve lesion in cardiac surgery. While the ability to obtain a
competent, durable tricuspid repair has improved in recent
years, the surgical treatment of the congenitally abnormal
tricuspid valve is still considered palliative because many
patients require more than one operation in their lifetime.
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